Takayasu's arteritis: A study of 104 Italian patients.

OBJECTIVE: Takayasu's arteritis (TA) is a rare vasculitis. The Italian Takayasu's Arteritis study group was established with the aim to describe a large cohort of patients. METHODS: Data were collected by means of an ad hoc form. Demographic information, clinical history, vascular findings, treatment, risk factors, and comorbidities were analyzed. RESULTS: Data of 104 patients were collected. The median delay in diagnosis was 15.5 months (range 0-325 months). Age at onset <15 years was associated with a higher probability, whereas elevated erythrocyte sedimentation rate with a lower probability, of a delay in diagnosis. The majority of patients experienced nonspecific signs and symptoms indicative of an inflammatory disease in the early phase. Among vascular involvement, stenosis was the most frequent lesion, being present in 93% of patients, followed by occlusion (57%), dilatation (16%), and aneurysm (7%). Glucocorticoids were the mainstay of treatment in our series; however, treatment with cytotoxic agents was required in about half of the patients. Fifty-two patients underwent at least 1 surgical procedure. The main indications for intervention were renal vascular hypertension, cerebral hypoperfusion, and limb claudication. CONCLUSION: As with many rare diseases, delay in diagnosis is an important issue for patients with TA. The increasing occurrence of vascular lesions along with the disease progression put to question the long-term effectiveness of contemporary treatment. These data may be helpful in increasing physicians' awareness to prevent diagnosis delay, update guidelines, and plan future research projects.

Ventilation-perfusion lung scan for the detection of pulmonary involvement in Takayasu's arteritis.

The aim of study was to analyse ventilation and perfusion (V/Q) lung scan findings in a series of Italian patients with Takayasu's arteritis. Eighteen consecutive patients underwent V/Q lung planar scintigraphy and single-photon emission tomography (SPET). Before perfusion scan acquisition was started, a first-pass study with (99m)Tc-macroaggregates of albumin was performed to assess the right ventricular ejection fraction (RVEF). All patients had normal chest X-rays and were symptom free at the time of the investigation. They also underwent echocardiography to evaluate pulmonary artery pressure and in 13 patients respiratory function tests were performed. In four patients, perfusion lung scan was repeated after 1 year. In 10/18 patients (55.5%), 43 unmatched lobar, segmental or subsegmental perfusion defects were found on planar images; ventilation scintigraphy was normal in all cases. On SPET images, 55 defects were found; no defects were found with SPET in the remaining patients who had normal planar images. All patients had normal RVEF and 5/13 patients had mild restrictive-obstructive lung disease. The pulmonary artery pressure was increased in two patients with perfusion defects. In the four patients who had repeat scintigraphy, all defects remained unchanged. The prevalence of lung perfusion abnormalities observed in Italian patients with Takayasu's arteritis is within the range of values reported in other countries, and V/Q planar scintigraphy is sufficient for the screening of patients.

Takayasu's arteritis: a changing disease.

Takayasu's arteritis (TA) is a chronic, giant-cell vasculitis of unknown etiology, which primarily involves the aorta, its main branches and coronary and pulmonary arteries. This review focuses on the epidemiological, diagnostic, and clinical aspects of the disease, which have been changed since the first description made by Mikito Takayasu in 1908. The article also summarizes the data collected by the Italian Registry of TA in the period 1993-1998.

Non-invasive assessment of pulmonary artery involvement in Takayasu's arteritis.

OBJECTIVE: To evaluate pulmonary involvement in Italian patients with Takayasu's arteritis (TA). METHODS: A prospective analysis of 15 Italian patients with TA was carried out, including evaluation by perfusion and ventilation lung scintigraphy (planar and tomographic), standard chest X-ray, spirography and color-doppler echocardiography. All the patients were free of respiratory symptoms when examined. RESULTS: In all patients standard chest X-rays and ventilation scintigraphies were normal. 9/15 patients showed unmatched segmental perfusion defects (41 by planar evaluation vs. 48 by SPET). The number of defects was greater in the right lung than in the left (26 vs 18), with a higher frequency of moderate or large defects. Thirteen patients underwent spirography, which proved to be abnormal in 5 cases. Two of these patients were also positive on scintigraphy. No patient showed alterations attributable to TA on color-doppler echocardiography, except for 3 patients with mild to moderate aortic valve regurgitation. CONCLUSIONS: Our results show that vascular pulmonary involvement is frequent in TA (60% of cases) even in the absence of clinical signs. The planar image, simpler than the SPET to acquire, was sufficient to make an accurate diagnosis. Italian patients seem to show a pattern of extrapulmonary and pulmonary vascular involvement very similar to that reported in Japanese subjects, and different from that observed in other ethnic groups.

Takayasu's arteritis in Italian patients.

OBJECTIVE: To evaluate the clinical features, angiographic findings and evolution of Takayasu's arteritis in Italian patients. METHODS: Retrospective analysis of the case records of 27 Italian patients with Takayasu's arteritis, all meeting the 1990 ACR criteria for classification of this vasculitis, with a median disease duration of 9.5 years. RESULTS: The patterns of arterial involvement and the frequencies of systemic and specific symptoms were similar, with some exceptions, to those reported in the literature. In our patients weight loss was rare, the incidence of respiratory symptoms and seizures was low, and no clinical manifestations of intestinal involvement were reported. All of the patients were treated with antiplatelet or anticoagulant medications, and 14 were also treated with corticosteroids and immunosuppressants; a stabilization of the vascular involvement was seen in most of them. Thirteen patients underwent surgery, and 5 had percutaneous transluminal renal angioplasties. Five pregnancies were observed, without complications. Two patients died, one after an angiographic evaluation and one for reasons unrelated to the disease. CONCLUSIONS: Takayasu's arteritis is heterogeneous in presentation, evolution and response to therapy. In our series of patients the disease seems to have showed a more favorable course than that previously reported. This can be attributed to the effectiveness of therapy, but also, at least partially, to the better prognosis that the disease seems to have for Western than for Eastern subjects.